Fay, in 1927, first used the term "carotidynia"
to refer to the tenderness of the bifurcation of
the carotid artery, sometimes observed in
patients with "atypical facial neuralgia." He
believed that the pain arose in the common
carotid sheath and reported satisfactory
remission of symptoms following carotid sheath
resection and cervical sympathectomy. In its
early description, carotidynia was a mere
physical sign of tenderness, and was later
referred to as the "Fay test. " However, over
the years, it changed into a distinct syndrome,
particularly when Roseman, in 1967, designated
it as "a distinct entity" characterized by two
cardinal signs: unilateral neck pain and
tenderness of the carotid artery. There were
hundreds of cases of carotidynia reported in the
1960s and 1970s, with two main types, acute and
chronic. More recently, acute idiopathic
carotidynia was accepted as a valid entity by
the International Headache Society (IHS)
Classification Committee with the following four
diagnostic criteria: "(A) At least one of the
following overlying the carotid artery: (1)
tenderness, (2) swelling, (3) increased
pulsations. (B) Appropriate investigations do
not reveal structural abnormality. (C) Pain over
the affected side of the neck. May project to
the ipsilateral side of the head. (D) A
self-limiting syndrome of less than 2 weeks
duration."

In the last 5 years, however, carotidynia
seems to have vanished almost completely from
the medical literature and, in our 20-year
experience with headache patients, we have never
seen a case of carotidynia as defined by the
IHS. It is therefore appropriate to ask, "Does
carotidynia exist or does it belong to
"neuromythology ?" To answer, we will discuss
the validity of the four main diagnostic
criteria for carotidynia proposed in the IHS
classification.

Neck pain.

Carotidynia is considered by most authors to
be a unilateral deep-seated neck pain radiating
to the ear and face and aggravated by head
movements, swallowing, chewing, coughing,
yawning, and
sneezing. However, many patients complain of
sore throat rather than neck pain, and in some
reports, neck pain was absent, replaced by pain
in the face or by headache.

The pain usually recurs on the same side but
may be alternating or bilateral. In one of the
classic anatomic cases, the patient did not
complain of any pain and had had "a bilateral
painless swelling at the level of carotid
bifurcation for several years." All degrees of
severity have been reported, from a mild
sensation of discomfort to an excruciating pain,
with most of the cases being moderate or severe.
The pain has been variously described as
throbbing, dull, stabbing, or pulling. The pain
of carotidynia, as reported in the literature,
is thus highly variable in its location, type,
and severity. It has no specific characteristics
and no consistent pattern, and it can even be
absent.

Tenderness.

Typically, the carotid artery, particularly
near the bifurcation, is described as tender,
swollen, and hyperpulsatile; its palpation
invokes local pain and frequently reproduces the
facial and head pain of the spontaneous attack.
Tenderness over the course of the carotid artery
was considered diagnostic in most series such as
those of Roseman, with a positive Fay test in
all 33 patients; of Davies, who described six
patients with "extreme tenderness comparable to
that of temporal arteritis" or of Raskin and
Prusiner, who reported eight patients with
"prominent carotid pulsations and tenderness
and/or neck swelling." However, in other series,
tenderness was inconstant (73 of the 100
patients reported by Lovshin and 12 of the 40
patients described by Chiossone and Quiroga) or
even absent. Furthermore, tenderness in the
region of the carotid artery can be present in
numerous other conditions such as lymphadenitis,
giant cell arteritis, aphtous ulcers, migraine,
cluster headache, and carotid occlusion and dis
section. Thus, the presence of tenderness over
the carotid artery is neither constant nor
specific and cannot be considered diagnostic of
carotidynia.

Temporal profile.

There are two main patterns in the
literature acute, with a single episode lasting
an average of 11 days,and chronic, with years of
recurrent attacks. However, on close examination
of the published cases, it is impossible to
define patterns of evolution. First, in some of
the large classic series, the temporal profile
is either not mentioned or very poorly
described. Second, all varieties of temporal
profiles have been reported; attacks may last
minutes, hours days, weeks, or months and their
frequency is highly variable, going from a
single episode to numerous episodes recurring
over many years. Some patients have a "dull
aching background pain episodically becoming
throbbing" and others an "intractable head pain
present for many years." Thus, in carotidynia,
there is no typical pattern of evolution and,
although a number of cases have been reported
with "a self-limiting syndrome of less than 2
weeks duration" as described by the IHS
classification , there are reports of many other
forms, and none is more validated than the
others.

Appropriate investigations.

The description of the IHS criteria for
carotidynia includes the comment that "organic
disease of the carotid artery may cause a
similar picture and that appropriate
investigations should rule out conditions such
as giant cell arteritis, atherosclerosis,
thrombosis, intraluminal hemorrhage,
fibromuscular dysplasia, aneurysm, and
aneurysmal dissection" all recognized as
possible causes of carotidynia. Although it is
not explicitly indicated which investigations
should be performed, a proper vascular workup is
crucial to rule out many of these conditions.
This applies particularly to extracraniai
internal carotid artery dissections, which can
present as an isolated unilateral neck pain
spontaneously resolving in less than 2 weeks.
Such investigations include Duplex scanning,
magnetic resonance imaging and angiography, and
intra-arterial angiography. Furthermore, they
should be performed in the first few days
because of the possibility of rapid
recanalization in cases of carotid
dissection.

In the hundreds of published cases such
vascular investigations have rarely been done,
and in the few cases with carotid angiography,
the carotid artery was rarely normal. Thus, in a
surgical series of 10 cases (four with stroke or
TIA), an arterial lesion was consistently found:
carotid occlusion, atheromatous stenosis with an
intraplaque hemorrhage, aneurysms (probably
dissecting) with cystic medial necrosis, and
fibromuscular dysplasia. Chambers et a reported
seven similar cases of carotidynia with an
underlying carotid artery disorder (four with
stroke or TIA); two patients had a dissecting
aneurysm of the internal carotid artery, two an
atheromatous aneurysm of the common carotid
bifurcation, two an intraluminal clot, and one a
giant cell ateritis. In a series of eight cases
reported by Raskin and Prusiner, carotid
angiography was performed in two and was found
to be normal. The first patient had a 20-year
history of migraine and a 12-year history of
chronic dull pain in the angle of the right jaw
that improved with antimigraine drugs. The
second patient also had a "chronic carotidynia"
with a 4-year history of left-sided neck pain
(with occasional scintillating scotomata) that
disappeared with methysergide treatment. De
Vries et al reported a 47 year-old woman with a
4-month history of continuous pain and
tenderness of the right carotid bulb associated
with dysphagia and weight loss. All
investigations, including angiography, were
normal. At surgery, the carotid artery was
normal. The bulb was dissected and denervated
with an immediate relief of symptoms. This
unique case is difficult to interpret,
particularly since this patient had renovascular
hypertension and had been operated on for
bilateral fibromuscular stenosis of renal
arteries.

Proposed treatments.

Numerous treatments have been claimed to be
effective in carotidynia, such as antimigraine
drugs, steroids, aspirin, and surgery; this
obviously suggests a wide pathogenetic
heterogeneity.

A nonvalidated entity.

Thus, critical review of the relevant
literature shows that carotidynia is not a
validated entity. The two clinical signs on
which the diagnosis is based (neck pain and
tenderness) are neither constant nor specific,
and there is no consistent pattern of temporal
profile. What has been reported under this term
in the medical literature is a potpourri of many
conditions. Recurring attacks of carotidynia
with headache lasting a few days are likely
related to migraine, attacks lasting a few
miites suggest chronic paroxysmal hemicrania,
and regular daily attacks of 1 or 2 hours
duration point to cluster headache. A viral
etiology has been postulated but never
scientifically established in acute varieties
with a self-limited course. As indicated above,
carotid dissection should be ruled out in such
cases. Chronic varieties with a permanent
background pain probably have an important
psychological component. There are reports of
causes such as carotid artery diseases
(occlusion fibromuscular dysplasia or giant cell
or after carotid endarterectomy) but also
carotid body tumors, lymphadenitis, pharyngitis,
dental infection, local aphthous ulcers, or
malignant infiltration.

Conclusion.

Our review indicates (as already suggested
by Chambers et al that (1) idiopathic
carotidynia is not a valid entity, and (2)
carotidynia is simply a syndrome of a unilateral
neck pain with local tenderness that can be due
to numerous vascular and nonvascular causes, and
therefore does not necessarily imply a carotid
ongin. "Carotidynia" is a highly misleading term
that should preferably be expunged from the
medical nosology.